Objectives This study assessed the type, time course, and risk factors for right and left ventricular outflow tract reinterventions after the Ross procedure in a population of infants, children, and ...young adults. Methods Patients who underwent the Ross procedure between January 1995 and June 2004 were included (n = 121 consecutive patients). Kaplan–Meier and hazard analyses of right and left ventricular outflow tract reinterventions were performed, and predictors of reintervention were identified through multivariate analysis. Results The median age at the Ross procedure was 8.2 years (4 days to 34 years); 20% were aged less than 1 year. Half of the patients had isolated aortic valve disease; the other half had complex left-sided heart disease. Early mortality (<30 days) was 2.5% (n = 3). There were 2 late deaths (1.7%). Follow-up (median 6.5 years 2.5 months to 10.4 years) was available for 96% of survivors (n = 111). Right ventricular outflow tract reintervention (n = 22 in 15 patients) was performed 2.0 years (2.0 weeks to 9.8 years) after the Ross procedure because of stenosis in 19 of 22 cases. Freedom from right ventricular outflow tract reintervention at 8 years was 81%. Smaller homograft size was the strongest predictor ( P < .001) of right ventricular outflow tract reintervention. Left ventricular outflow tract reintervention (n = 15 in 15 patients) was performed 2.8 years (1.0 months to 11.6 years) after the Ross procedure because of severe neoaortic insufficiency in 10 of 15 patients. Freedom from left ventricular outflow tract reintervention at 8 years was 83%. Native pulmonary valve abnormalities ( P < .01), original diagnosis of aortic insufficiency ( P < .01), prior aortic valve replacement ( P = .01), and prior ventricular septal defect repair ( P = .04) predicted left ventricular outflow tract reintervention. Conclusions At midterm follow-up after the Ross procedure, interim mortality is rare. Neoaortic insufficiency and right ventricle to pulmonary artery conduit obstruction are common postoperative sequelae, requiring reintervention in one quarter of patients.
The Future of Cardiac Imaging Douglas, Pamela S., MD; Cerqueira, Manuel D., MD; Berman, Daniel S., MD ...
JACC. Cardiovascular imaging,
October 2016, Letnik:
9, Številka:
10
Journal Article
Recenzirano
Odprti dostop
Abstract The American College of Cardiology’s Executive Committee and Cardiovascular Imaging Section Leadership Council convened a discussion regarding the future of cardiac imaging among thought ...leaders in the field during a 2 day Think Tank. Participants were charged with thinking broadly about the future of imaging and developing a roadmap to address critical challenges. Key areas of discussion included: 1) how can cardiac imaging services thrive in our new world of value-based health care? 2) Who is the cardiac imager of the future and what is the role of the multimodality imager? 3) How can we nurture innovation and research in imaging? And 4) how can we maximize imaging information and optimize outcomes? This document describes the proceedings of this Think Tank.
Surgical and perioperative improvements permit earlier repair of partial and transitional atrioventricular septal defects (AVSD). We sought to describe contemporary outcomes in a multicenter cohort.
...We studied 87 patients undergoing primary biventricular repair of partial or transitional AVSD between June 2004 and February 2006 across seven North American centers. One-month and 6-month postoperative data included weight-for-age z-scores, left atrioventricular valve regurgitation (LAVVR) grade, residual shunts, and left ventricular ejection fraction. Paired methods were used to assess 6-month change.
Median age at surgery was 1.8 years; median weight z-score was -0.88. Median days for ventilation were 1, intensive care 2, and hospitalization 5, all independent of age, with 1 in-hospital death. At 1 month, 27% (16 of 73) had ejection fraction less than 55%; 20% (17 of 87) had significant LAVVR; 2 had residual shunts; 1 each had subaortic stenosis and LAVV stenosis. At 6 months (n = 60), there were no interim deaths, reinterventions, or new development of subaortic or LAVV stenosis. Weight z-score improved by a median 0.4 units (p < 0.001), especially for underweight children less than 18 months old. Left atrioventricular valve regurgitation occurred in 31% (change from baseline, p = 0.13), occurring more frequently in patients repaired at 4 to 7 years (p = 0.01). Three patients had ejection fraction less than 55%, and 1 had a residual atrial shunt.
Surgical repair for partial/transitional AVSD is associated with low morbidity and mortality, short hospital stays, and catch-up growth, particularly in underweight children repaired between 3 and 18 months of age. Left atrioventricular valve regurgitation remains the most common residual defect, occurring more frequently in children repaired after 4 years of age.
The outcome of biventricular (BV) repair for right-dominant unbalanced atrioventricular canal has remained poor, because it is difficult to predict left ventricular (LV) adequacy before surgery. Our ...aim was to determine whether preoperative echocardiographic parameters, specifically analysis of color inflow into the LV, would predict survival after BV repair in patients with right-dominant unbalanced atrioventricular canal. Subjects with right-dominant unbalanced atrioventricular canal diagnosed from 1994 to 2007 were included. The echocardiographic parameters were analyzed blinded to the palliation strategy and survival. The LV inflow index (LVII) was calculated as the secondary color inflow diameter indexed to the left atrioventricular valve (AVV) annulus diameter. Univariate analysis, survival analysis, and multivariate modeling with stepwise logistic regression were performed. Of the 45 subjects, 23 (51%) underwent single ventricle (SV) palliation and 22 (49%) underwent BV repair. Of the 23 who underwent SV palliation, 15 (65%) survived compared to 18 (82%) of 22 who underwent BV repair (p = 0.34). In the BV group, a greater LVII predicted survival (R2 = 0.46, p = 0.03). No subjects with a LVII <0.5 survived BV repair. Mortality in the BV group was associated with younger age at initial surgery (p <0.01) and abnormal left AVV morphology (p = 0.02). Of the BV subjects with a patent ductus arteriosus at the initial operation (n = 11), the nonsurvivors were more likely to have retrograde flow in the transverse arch (p <0.01). In the BV group, reoperation within 30 days of the initial repair was strongly associated with mortality (p <0.01). In conclusion, in cases of mild or moderate LV hypoplasia, a greater LVII predicted survival after BV repair in patients with right-dominant unbalanced atrioventricular canal. We propose incorporation of the LVII into the echocardiographic assessment of these patients.
Background Over 25% of patients with congenital heart disease are obese (OB) or overweight (OW). Unique factors such as activity restriction and early feeding practices may play a role. We evaluated ...a high-risk cohort predisposed to early coronary artery disease due to past surgery involving coronary artery reimplantation. Methods Patients at our institution who underwent the arterial switch or Ross operation were included. Data collection included chart review and activity and diet questionnaires. The proportion of OB/OW (body mass index ≥85%) was compared to national data. Factors associated with OB/OW were evaluated. Results A total of 106 patients were enrolled. Median age was 14.2 years (6.0-19.9 years); 69% were male. Similar to national data, 31% were OB/OW (vs 31%, P = nonsignificant NS). There was no difference comparing OB/OW to normal-weight patients in activity restriction (cardiologist documented 27% vs 27%; parent reported 38% vs 42%, P = NS), activity level (9.8 hours/week 1.7-41.2 vs 11.5 0.8-52.3, P = NS), or early feeding practices (formula vs breastfeeding, caloric fortification, tube feeding). OB/OW patients were more likely to have an OB parent (70% vs 37%, P = .01). Left ventricular mass index was higher in OB/OW patients (48.8 g/m2.7 24.4-120.6 vs 37.7 g/m2.7 16.5-85.1, P = .01). Conclusions Nearly one third of ASO and Ross patients were OB/OW. Obese/overweight was not found to be related to activity restriction or early feeding practices, but was associated with parental obesity. Obese or OW patients had higher left ventricular mass. Obesity and associated comorbidities may pose additional cardiovascular risk in this unique population who underwent coronary artery reimplantation in childhood.
Echocardiography is the imaging modality of choice for the evaluation of coronary artery (CA) abnormalities in Kawasaki disease. Small series have established high specificity and sensitivity for ...detecting abnormalities, yet visualization rates of individual CA segments and factors associated with success are unknown.
In the Pediatric Heart Network's randomized trial of primary steroid treatment for Kawasaki disease, echocardiograms were interpreted locally and by a core laboratory. Univariate and multivariate predictors of CA visualization by the local lab as determined by the core lab were explored, and agreement of CA size measured locally and by the core lab was assessed.
A total of 589 echocardiograms from 199 patients were obtained over 27 months. Visualization rates for the left main, proximal and distal left anterior descending, and proximal right CAs ranged from 91% to 98% but were lower for the distal right (65%), circumflex (86%), and posterior descending (54%) CAs. For the distal right and circumflex CAs, visualization rates improved over the course of the study (P<.05). In multivariate analysis, local center, CA segment, and time from study start to echocardiography were independent predictors of visualization (all P values<.001). For segments for which visualization rates varied by center, higher percentage visualization was associated with larger center volume (P=.001). Routine sedation use was also associated with higher visualization rates.
Successful CA visualization in Kawasaki disease is associated with the segment being evaluated and is influenced by center volume and sedation use. Increased visualization rates over time suggest a learning curve and underscore the value of core lab oversight in pediatric multicenter trials.
Background Mitral regurgitation is the most common indication for reoperation in children following repair of atrioventricular septal defect (AVSD). We hypothesized that angiotensin-converting enzyme ...inhibitor therapy would decrease the severity of mitral regurgitation and limit left ventricular volume overload in children following AVSD repair. Methods The Pediatric Heart Network designed a placebo-controlled randomized trial of enalapril in this population. The primary aim was to test the effect of enalapril on the change in left ventricular end-diastolic dimension body surface area–adjusted z score. Before the launch of the trial, a feasibility study was performed to estimate the number of patients with at least moderate mitral regurgitation following AVSD repair. Trial experience Seventeen months after the start of the study, 349 patients were screened, 8 were trial eligible, and only 5 were enrolled. The study was subsequently terminated because of low patient accrual. Several factors led to the problems with patient accrual, including (1) the use of criteria to assess disease severity in the feasibility study that were not identical to those used in the trial, (2) failure to achieve equipoise for the study among clinicians and referring physicians, (3) reliance on methodology developed in adult populations with different disease mechanisms, and (4) absence of adequate data to define the natural history of the disease process under study. Progress in the treatment of children with cardiovascular disease will depend on the future of multicenter collaborative clinical trials. The lessons learned from this study may contribute to improvements in this research.
Background Wyman W. Lai, MD, MPH, and Victoria L. Vetter, MD, MPH. The Pediatric Heart Network (PHN), funded under the U.S. National Institutes of Health–National Heart, Lung, and Blood Institute ...(NIH–NHLBI), includes two Clinical Research Skills Development (CRSD) Cores, which were awarded to The Children's Hospital of Philadelphia and to the Morgan Stanley Children's Hospital of New York–Presbyterian. To provide information on how to develop a clinical research career to a larger number of potential young investigators in pediatric cardiology, the directors of these two CRSD Cores jointly organized a one-day seminar for fellows and junior faculty from all of the PHN Core sites. The participants included faculty members from the PHN and the NHLBI. The day-long seminar was held on April 29, 2009, at the NHLBI site, immediately preceding the PHN Steering Committee meeting in Bethesda, MD. Methods The goals of the seminar were 1) to provide fellows and early investigators with basic skills in clinical research 2) to provide a forum for discussion of important research career choices 3) to introduce attendees to each other and to established clinical researchers in pediatric cardiology, and 4) to publish a commentary on the future of clinical research in pediatric cardiology. Results The following chapters are compilations of the talks given at the 2009 PHN Clinical Research Skills Development Seminar, published to share the information provided with a broader audience of those interested in learning how to develop a clinical research career in pediatric cardiology. The discussions of types of clinical research, research skills, career development strategies, funding, and career management are applicable to research careers in other areas of clinical medicine as well. Conclusions The aim of this compilation is to stimulate those who might be interested in the research career options available to investigators.
Background Friedreich ataxia (FRDA) is commonly associated with hypertrophic cardiomyopathy, but little is known about its frequency, severity, or treatment. In this 6-month randomized, double-blind, ...controlled study, we sought to determine whether idebenone improves cardiac measures in FRDA. Methods Seventy pediatric subjects were treated either with idebenone (450/900 mg/d or 1,350/2,250 mg/d) or with placebo. Electrocardiograms (ECGs) were assessed at each visit, and echocardiograms, at baseline and week 24. Results We found ECG abnormalities in 90% of the subjects. On echocardiogram, 81.4% of the total cohort had left ventricular (LV) hypertrophy, as measured by increased LV mass index–Dubois, and the mean ejection fraction (EF) was 56.9%. In linear regression models, longer PR intervals at baseline were marginally associated with longer GAA repeat length ( P = .011). Similarly, GAA repeat length did not clearly predict baseline EF ( P = .086) and LV mass by M-mode ( P = .045). Left ventricular mass index, posterior wall thickness, EF, and ECG parameters were not significantly improved by treatment with idebenone. Some changes in echocardiographic parameters during the treatment phase correlated with baseline status but not with treatment group. Conclusions Idebenone did not decrease LV hypertrophy or improve cardiac function in subjects with FRDA. The present study does not provide evidence of benefit in this cohort over a 6-month treatment period.
Growth hormone and its mediator, insulinlike growth factor 1 (IGF-1), are key determinants of growth in children and young adults. As patients with Fontan physiology often experience diminished ...longitudinal growth, we sought to describe IGF-1 levels in this population and to identify factors associated with IGF-1 deficiency. Forty-one Fontan subjects ≥5 years were evaluated in this cross-sectional study. Age- and gender-specific height Z scores were generated using national data. Laboratory testing included IGF-1 and brain natriuretic peptide (BNP) levels. IGF-1 levels were converted to age-, gender-, and Tanner stage–specific Z scores. BNP levels were log transformed to achieve a normal distribution (log-BNP). Medical records were reviewed for pertinent clinical variables. Predictors of IGF-1 Z score were assessed through the Student t test and Pearson's correlation. Median age was 11.1 years (range 5.1 to 33.5 years), and time from Fontan was 8.2 years (1.1 to 26.7). Mean height Z score was −0.2 ± 0.9 with a mean IGF-1 Z score of −0.1 ± 1.3. There was no association between IGF-1 Z score and height Z score. Longer interval since Fontan ( R = −0.32, p = 0.04), higher log-BNP ( R = −0.40; p = 0.01), and lower indexed systemic flow on cardiac magnetic resonance ( R = 0.55, p = 0.02) were associated with lower IGF-1 Z scores. In conclusion, in this cohort with Fontan physiology, higher BNP and lower systemic flow were associated with lower IGF-1 Z score. Longitudinal studies are needed to determine if these relations represent a mechanistic explanation for diminished growth in children with this physiology and with other forms of congenital heart disease.
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